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Papers by mohammadreza modaresi

Research paper thumbnail of Comparing effects of low glycemic index/high-fat, high-calorie diet and high-fat, high-calorie diet on cytokine levels of patients with cystic fibrosis: A randomized controlled clinical trial

European Cytokine Network

The importance of the host inflammatory response, as a central pathological feature of cystic fib... more The importance of the host inflammatory response, as a central pathological feature of cystic fibrosis, is well recognized. Additionally, hyperglycemia can induce an immune response and consecutively may exacerbate symptoms of this disease. Hence, adherence to a low glycemic index diet, through normalizing blood glucose levels, may reduce inflammation in patients with this disease. This study aimed to compare effects of a low glycemic index/high-fat, high-calorie diet and routine high-fat, high-calorie diet on inflammatory biomarkers in patients with cystic fibrosis. In this randomized clinical trial, 44 children and adolescents with cystic fibrosis were randomly assigned to receive for three months either a high-fat, high-calorie diet (n = 22) or a low glycemic index/high-fat, high-calorie diet (n = 22) with similar calorie and macronutrients composition to the control diet. Patients in first arm were allowed to use all sources of carbohydrates with different glycemic indices, whereas those in another arm consumed carbohydrates from low glycemic index sources. Serum levels of the pro-inflammatory cytokines IL-6, IL-17A, and IFNg, and the antiinflammatory cytokine IL-10 were measured at baseline and after the end of the trial. There were significant differences between groups for IL-6 (P = 0.02) and IL-17 (P = 0.01), in favor of the low glycemic diet, but no between-group differences were detected in IL-10 and IFN-γ. Although serum levels of IL-17 were reduced in both the groups as compared with the baseline values, this reduction was only significant in the group assigned to the low glycemic diet (P= 0.007), In addition, IL-6 serum levels decreased and those of IL-10 increased significantly as compared with the baseline values in the low glycemic diet (P= 0.01). It seems that adherence to a low glycemic index/high-fat, high-calorie diet for three months can improve some inflammatory biomarkers in children and adolescents with cystic fibrosis compared with the high-fat, high-calorie diet.

Research paper thumbnail of The value of bronchocele attenuation in pulmonary computed tomography in assessment of allergic bronchopulmonary aspergillosis in the background of cystic fibrosis: A cross-sectional study

Annals of Medicine and Surgery

Research paper thumbnail of Relationship Between the Burden of Care and Fear of Disease Progression in Mothers of Children with Cystic Fibrosis

International journal of health sciences

Objectives: Aim of this study was determining the relationship between burden of care and fear of... more Objectives: Aim of this study was determining the relationship between burden of care and fear of disease progression in mothers of children with cystic fibrosis. Methodology: 142 mothers of children with cystic fibrosis (CF) participated in this correlational study. Data were collected with sequential method. The researchers employed the self-report Zarit burden of care and fear of disease progression questionnaire in parents of children with CF. Mothers answered the questioners separately. Data were analyzed with statistical analysis of variance and independent t-test. Results: According to the results, the mean maternal burden of care was 34.12 ± 69.77, and the mean fear of disease progression was 32.12 ± 92.11. The burden of care had the highest regression effect on fear of disease progression in mothers of children with CF (β = 0.896). With an increase of one unit of standard deviation in the burden of care, an increase of 0.896 units occurred in fear of disease progression in...

Research paper thumbnail of The Value of Sputum Polymerase Chain Reaction for Detection of Nontuberculous Mycobacteria in Cystic Fibrosis Patients with Negative Nontuberculous Mycobacteria Sputum Culture

Medical Journal of The Islamic Republic of Iran, 2022

Background value of Polym Methods: Th with an NTM n sequence. Results: In to for NTM. Amo Conclus... more Background value of Polym Methods: Th with an NTM n sequence. Results: In to for NTM. Amo Conclusion: always under c

Research paper thumbnail of Cough in adolescent with cystic fibrosis, from nightmare to COVID-19 stigma: A qualitative thematic analysis

Journal of Pediatric Nursing, 2022

Purpose Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common... more Purpose Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common symptom. This study explored the experiences of adolescents with CF in Iran during the COVID-19 pandemic in relation to their cough. Design and methods In this qualitative study, we conducted 32 semi-structured interviews with 21 adolescents with CF. We analyzed the data thematically. Results We identified three main themes among adolescents with CF in relation to coughing: 1. Cough is a permanent companion; 2. Coughing raises fear of double stigma; 3. Patients' individualized coping strategies to deal with coughing. Participants complained that cough interrupted daily tasks and sleep, drew unwanted attention in public places, and elicited questions about whether they were COVID-19 patients or substance users—both highly stigmatized identities. Conclusion Although coughing is a protective mechanism for CF patients, frequent coughing often causes major challenges, particularly during the COVID pandemic, when people were acutely sensitive and aware about coughing. During the COVID-19 pandemic, in addition to taking care of themselves and managing the disease, CF patients therefore had to also overcome issues related to social stigma and isolation. Practice implications Healthcare workers play an important role in increasing public awareness about CF and its symptoms, including cough. During the pandemic, healthcare workers can help reduce the stigma of coughing through public education. Healthcare workers can actively communicate with patients to identify severe and ineffective cases of cough due to exacerbation of the disease and refer them to a specialist.

Research paper thumbnail of The correlation between 6-min walk test and respiratory parameters in children with cystic fibrosis

Irish Journal of Medical Science (1971 -), 2021

Background The 6-min walk test (6MWT) is a sub-maximal exercise test and has been widely used for... more Background The 6-min walk test (6MWT) is a sub-maximal exercise test and has been widely used for evaluating of exercise capacity of patients with cystic fibrosis (CF) in recent years. Few studies have examined the relationship between 6MWT and parameters used to assess the severity of the disease in children with CF. In this study, we have examined this relationship to find out if 6MWT can be a marker of the severity of cystic fibrosis. Methods A cross-sectional study was done to analyze the correlations among spirometry parameters, body mass index (BMI), chest tomography (CT), and 6MWT. CF patients, aged 7–14 years, were involved. Results Seventy-six patients, 32F/44M, mean age 10.49 ± 3.18 years, were studied. The mean distance in 6MWT was 447 ± 84.1. The following correlations versus distance were found: FEV1 ( r = 0.255, p = 0.026), FVC( r = 0.285, p = 0.013), FEF25–75% ( r = 0.546, p < 0.001), BMI ( r = 0.163, p = 0.160), and CT ( r = 0.075, p = 0.520).The following correlations versus O 2 saturation (SpO 2 ) decline were found: FEV1 ( r = −0.393, p < 0.001), FVC ( r = −0.431, p < 0.001), FEF25–75% ( r = −0.296, p = 0.010), BMI ( r = 0.042, p = 0.721), and CT ( r = −0.196, p = 0.090). There was a significant correlation between 6MWT (distance and SpO 2 decline) and pulmonary function test. There was no significant correlation between BMI, chest CT, and 6MWT. Conclusions 6MWT can be applied beside spirometry and chest CT for CF patients follow up.

Research paper thumbnail of Farber disease: report of three cases with joint involvement mimicking juvenile idiopathic arthritis

Journal of Musculoskeletal & Neuronal Interactions, 2019

Farber disease is a rare recessive autosomal disorder presented with three main features of joint... more Farber disease is a rare recessive autosomal disorder presented with three main features of joint involvement, subcutaneous nodules and hoarseness. Hereby we describe three new cases of Farber disease. All three cases were first misdiagnosed as juvenile idiopathic arthritis (JIA) due to the presentation of joint swelling. Addition of hoarseness and subcutaneous nodules to the initial joint swelling questioned the diagnosis of JIA and further evaluations led to the diagnosis of Farber disease. The first case was a 4-year old girl in whom a novel genetic mutation in ASAH1 gene was found. The second patient was a 4-year old girl presented with joint swelling at 7 month of age. The third patient was a 9-month boy complicated with severe respiratory distress. All patients were treated with symptomatic and supportive care. Two cases died due to respiratory failure and infection, but one patient follow up for 2 years after diagnosis. Farber disease should be considered as differential diag...

Research paper thumbnail of The Prevalence of Obesity, Technological Device Usage, Physical Activity and their Relationship with Spirometry Indicators among Children in Isfahan City, Iran

International Journal of Pediatrics, 2018

Background: The decrease in physical activities following increased usage of computer and digital... more Background: The decrease in physical activities following increased usage of computer and digital games has led to serious health consequences in children. This study investigates the prevalence of obesity, cellphone and computer usage and physical activity levels and their relationship with spirometry indicators in Iranian children. Materials and Methods: This is a cross-sectional study during 2013 to 2014 on high-school students in Isfahan, Iran. Sample size determined 1,690 students and sampling performed from 10 girls and 10 boy's high schools, based on multi-staged cluster randomized scheme. A research-made questionnaire was developed to complete by students interview and also contact with teachers and parents and measuring height and weight by researchers. If the asthma was probable (based symptoms and examined by physician) the spirometry was performed. Results: Overall 1,622 high-school students with the average age of 12.9 (+ 1.1) years were recruited. Prevalence of ob...

Research paper thumbnail of Genetic modification of cystic fibrosis with ΔF508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells

Iranian Journal of Basic Medical Sciences, 2021

Objective(s): Cystic fibrosis (CF) is an inherited autosomal recessive disease that is caused by ... more Objective(s): Cystic fibrosis (CF) is an inherited autosomal recessive disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The present study aimed to investigate the genetic modification of CF with ΔF508 mutation of the CFTR gene using CRISPR in peripheral blood mononuclear cells (PBMCs). Materials and Methods: Two single guide RNAs were designed to target sequences in the CFTR gene. The transfection efficiency of PBMC cells was examined through evaluation of green fluorescent protein (GFP) expression using fluorescent microscopy. Moreover, a sgRNA-Cas9 plasmid was tested to target the CFTR gene. The ΔF508 gene modification was evaluated and confirmed by PCR and Sanger sequencing methods. Results: Our results indicate the feasibility of site-specific gene targeting with the CRISPR/Cas9 system. 33% of the samples were corrected using CRISPR in mutant locus and confirmed by sequence blast at NCBI databases and primers outside the...

Research paper thumbnail of Infection Detection in Cystic Fibrosis Patients based on Tunable Q-factor Wavelet Transform of Respiratory Sound Signal and Ensemble Decision

Scientia Iranica, 2020

Most adult Cystic Fibrosis (CF) patients frequently suffer from Pseudomonas aeruginosa (PA) infec... more Most adult Cystic Fibrosis (CF) patients frequently suffer from Pseudomonas aeruginosa (PA) infection, which is strongly associated with inflammation, lung destruction, and increased mortality. Diagnosis of PA infection in the primary stage is essential to initiate the treatment and reduce the risk of chronic infection. Sputum culture is the gold standard for infection detection, but it is time-consuming. The objective of this study was to suggest and examine a method to decide about PA infection status in CF patients based only on their respiratory sound. Respiratory sounds were recorded from 36 CF patients. Some features which were generated from Tunable Qfactor wavelet transform(TQWT) components, were investigated. The features were fed into Support Vector Machine and also Ensemble classifier. The proposed method achieved an accuracy of 90.3% in identifying PA infection in CF patients. Furthermore, the probability of categorizing respiratory sounds as PA CF decreased significantly after the treatment of PA infection(P-value<0.003). Moreover, the method had a satisfactory performance in the presence of noises and artifacts. The developed method represents a novel approach to the diagnosis of PA infection in CF patients based only on respiratory sound signals, which is a necessary and innovative approach for early diagnosis of PA infection.

Research paper thumbnail of Nebulized Azithromycin Versus Oral Azithromycin as Anti-Inflammatory Therapy in Children with Cystic Fibrosis: A Prospective Randomized Open-Label Trial

Iranian Journal of Pediatrics, 2019

Background: Oral azithromycin is recommended as anti-inflammatory therapy for patients with cysti... more Background: Oral azithromycin is recommended as anti-inflammatory therapy for patients with cystic fibrosis (CF) 6 years of age and older to improve lung function and reduce exacerbations. Objectives: We evaluated the short-term efficacy and safety of nebulized azithromycin as anti-inflammatory therapy in children with CF chronically infected with Pseudomonas aeruginosa. Methods: In a randomized prospective study, children aged 8-18 years with clinically stable CF and FEV1 25%-75% predicted with no recent use of oral, intravenous or inhaled antipseudomonal antibiotics and azithromycin were randomized to receive either nebulized azithromycin (70 mg daily) or oral azithromycin 3 times per week for 28 days. Primary endpoint was changes in pulmonary function (FEV1). Secondary outcomes included changes in Pseudomonas aeruginosa (PA) colonization characteristics (count, phenotype), quality of life and weight. Results: A total of 280 patients were screened, of whom 60 were found eligible. Forty five patients (25 in the nebulized group) and 20 in the oral group) completed the study. After 28 days of treatment, nebulized azithromycin was significantly associated with improvements in FEV1 % predicted, quality of life measure and weight. Also PA count was significantly decreased. Importantly, improvements of FEV1 % predicted, quality of life, weight and the decline in PA count were statistically significantly greater with nebulized azithromycin compared with oral azithromycin. No significant difference was observed in PA phenotype in either treatment arms. Conclusions: Nebulized azithromycin is associated with a significant improvement in lung function, PA count, quality of life and weight in clinically stable CF children with chronic PA infection. The improvements observed were greater compared with oral azithromycin. Nebulized azithromycin could be suggested as a new therapeutic strategy for this life-limiting disease. Further clinical trials with novel nebulizer formulation of azithromycin and large number of participants are needed to further assess the efficacy, safety and sustained effect of this new therapeutic approach in children with CF.

Research paper thumbnail of Indications and Complications of Flexible Fiberoptic Bronchoscopy in Children: A 5-Year Experience at a Tertiary Care Hospital in Iran

Iranian Journal of Pediatrics, 2020

Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therap... more Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therapeutic modality in the evaluation of respiratory disorders in pediatric population. Objectives: The study aims to highlight common indications and risk of complications associated with bronchoscopy in our population. Methods: This retrospective evaluation was performed in all patients that underwent flexible bronchoscopy at Children's Medical Center (affiliated to Tehran University of Medical Sciences) between April 2011 and September 2016. Results: Of 800 bronchoscopies, 574 (71.7%) were performed for diagnostic and 226 (28.2%) for therapeutic purposes. Major indications included radiographic abnormalities (30%), a foreign body or suspected foreign body (28.5% of all FFBs) and stridor or wheezing (25% of all FBB). The incidence of major complications associated with FFBs was 0.87%. The most frequent complication was pneumothorax, followed by lung hemorrhage, and respiratory failure. Conclusions: Our findings support early intervention and utilization of bronchoscopy in the pediatric population with variable respiratory complaints.

Research paper thumbnail of Evaluation of adherence to medication treatment in pediatric patients with cystic fibrosis; a cross-sectional study

Cystic fibrosis, 2019

Background: Cystic fibrosis is a complex, multi-system impairment with poly pharmacy which affect... more Background: Cystic fibrosis is a complex, multi-system impairment with poly pharmacy which affects both children and their parents. Adherence to treatment is essential to optimize CF disease management. Aims and Objectives: The aim of the present study was to evaluate the medication adherence and indeed identify the factors affecting adherence in this population. Methods: An observational prospective study was conducted to recruited CF outpatient at the CF clinic of Children’s Medical Center in Iran. Children with CF and their parents were consecutively recruited. Baseline data, medical history, clinical outcome data, were collected via interview and review of medical notes. The Morisky Medication Adherence scale (MMAS) was used to measure adherence. Results: Three hundred patients with CF (age 1-25 years, mean 7.6 years, 58.7% males) and parent pairs were recruited. The mean score of MMAS in pulmonary, antibiotics, gastrointestinal and supplement medications was 5.1, 4.6, 4.1 and 3.3 respectively. According to the categorization of Morisky Scale, 58% of patients who received inhaler medications and 65% of patients who received antibiotics had a low adherence (lower than 6 scores). High adherence was associated with child age, parental higher educational degree and their beliefs, duration of illness and frequency of healthcare visit ((P=0.001). Conclusion: The results indicated that the non-adherence was very common in patients with CF. Our results suggest the need for educational interventions and planned patient monitoring program in patients with CF to optimize management and health outcomes.

Research paper thumbnail of Segment by segment lavage as an excellent alternative treatment in pulmonary alveolar proteinosis in a 3 years old boy

Research paper thumbnail of Is FEV1 associated with quality of life in children with cystic fibrosis? An original article

Immunopathologia Persa, 2019

Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life ... more Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis. Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44) were boys. The mean t...

Research paper thumbnail of First Cystic Fibrosis Patient Registry Annual Data Report-Cystic Fibrosis Foundation of Iran

ACTA MEDICA IRANICA, 2019

Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortali... more Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aimed to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced expiratory volume in one second (FEV1) was measured, and the comparative demographic, clinical, and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and severe (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%). Regarding the complex nature of CF and the necessity of constant monitoring of patients during the lifespan , the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients' data can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population.

Research paper thumbnail of Efficacy and safety of oral sildenafil in cystic fibrosis children with mild to moderate lung disease

Pediatric Pulmonology, 2019

Background: Airway inflammation due to chronic infection is the leading cause of respiratory fail... more Background: Airway inflammation due to chronic infection is the leading cause of respiratory failure and death in most of patients with cystic fibrosis (CF). There is some evidence about anti-inflammatory activity of phosphodiesterase inhibitors in adult patients with CF. This study was designed to evaluate the efficacy, safety, and tolerability of sildenafil (a phosphodiesterase inhibitor drug) in children with CF. Method: This uncontrolled before-after study was conducted on 20 children with CF (mean age 14 ± 2.8 years, 50% male) with mild to moderate lung disease who were referred to CF clinic of Imam Hossein hospital in Isfahan, Iran. The patients received oral sildenafil (1 mg/kg p.o tid for 3 months). Changes in spirometric values, maximal exercise capacity, and patient-reported health by using the cystic fibrosis questionnaire-revised (CFQ-R) were evaluated before and after treatment.

Research paper thumbnail of A new model-based framework for lung tissue segmentation in three-dimensional thoracic CT images

Signal, Image and Video Processing, 2017

A new framework for model-based lung tissue segmentation in three-dimensional thoracic CT images ... more A new framework for model-based lung tissue segmentation in three-dimensional thoracic CT images is proposed. In the first stage, a parametric model for lung segmenting surface is created using shape representation based on level sets method. This model is constituted by the sum of a mean distance function and a number of weighted eigenshapes. Consequently, unlike the other model-based segmentation methods, there is no need to specify any marker point in this model. In the second stage, the segmenting surface is varied so as to be matched with the binarized input image. For this purpose, a region-based energy function is minimized with respect to the parameters including the weights of eigenshapes and coefficients of a three-dimensional similarity transform.

Research paper thumbnail of LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells

Acta medica Iranica, 2016

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described... more LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to dat...

Research paper thumbnail of Primary ciliary dyskinesia in six patients with bronchiectasis

Pneumonologia i alergologia polska, 2016

Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. ... more Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and histor...

Research paper thumbnail of Comparing effects of low glycemic index/high-fat, high-calorie diet and high-fat, high-calorie diet on cytokine levels of patients with cystic fibrosis: A randomized controlled clinical trial

European Cytokine Network

The importance of the host inflammatory response, as a central pathological feature of cystic fib... more The importance of the host inflammatory response, as a central pathological feature of cystic fibrosis, is well recognized. Additionally, hyperglycemia can induce an immune response and consecutively may exacerbate symptoms of this disease. Hence, adherence to a low glycemic index diet, through normalizing blood glucose levels, may reduce inflammation in patients with this disease. This study aimed to compare effects of a low glycemic index/high-fat, high-calorie diet and routine high-fat, high-calorie diet on inflammatory biomarkers in patients with cystic fibrosis. In this randomized clinical trial, 44 children and adolescents with cystic fibrosis were randomly assigned to receive for three months either a high-fat, high-calorie diet (n = 22) or a low glycemic index/high-fat, high-calorie diet (n = 22) with similar calorie and macronutrients composition to the control diet. Patients in first arm were allowed to use all sources of carbohydrates with different glycemic indices, whereas those in another arm consumed carbohydrates from low glycemic index sources. Serum levels of the pro-inflammatory cytokines IL-6, IL-17A, and IFNg, and the antiinflammatory cytokine IL-10 were measured at baseline and after the end of the trial. There were significant differences between groups for IL-6 (P = 0.02) and IL-17 (P = 0.01), in favor of the low glycemic diet, but no between-group differences were detected in IL-10 and IFN-γ. Although serum levels of IL-17 were reduced in both the groups as compared with the baseline values, this reduction was only significant in the group assigned to the low glycemic diet (P= 0.007), In addition, IL-6 serum levels decreased and those of IL-10 increased significantly as compared with the baseline values in the low glycemic diet (P= 0.01). It seems that adherence to a low glycemic index/high-fat, high-calorie diet for three months can improve some inflammatory biomarkers in children and adolescents with cystic fibrosis compared with the high-fat, high-calorie diet.

Research paper thumbnail of The value of bronchocele attenuation in pulmonary computed tomography in assessment of allergic bronchopulmonary aspergillosis in the background of cystic fibrosis: A cross-sectional study

Annals of Medicine and Surgery

Research paper thumbnail of Relationship Between the Burden of Care and Fear of Disease Progression in Mothers of Children with Cystic Fibrosis

International journal of health sciences

Objectives: Aim of this study was determining the relationship between burden of care and fear of... more Objectives: Aim of this study was determining the relationship between burden of care and fear of disease progression in mothers of children with cystic fibrosis. Methodology: 142 mothers of children with cystic fibrosis (CF) participated in this correlational study. Data were collected with sequential method. The researchers employed the self-report Zarit burden of care and fear of disease progression questionnaire in parents of children with CF. Mothers answered the questioners separately. Data were analyzed with statistical analysis of variance and independent t-test. Results: According to the results, the mean maternal burden of care was 34.12 ± 69.77, and the mean fear of disease progression was 32.12 ± 92.11. The burden of care had the highest regression effect on fear of disease progression in mothers of children with CF (β = 0.896). With an increase of one unit of standard deviation in the burden of care, an increase of 0.896 units occurred in fear of disease progression in...

Research paper thumbnail of The Value of Sputum Polymerase Chain Reaction for Detection of Nontuberculous Mycobacteria in Cystic Fibrosis Patients with Negative Nontuberculous Mycobacteria Sputum Culture

Medical Journal of The Islamic Republic of Iran, 2022

Background value of Polym Methods: Th with an NTM n sequence. Results: In to for NTM. Amo Conclus... more Background value of Polym Methods: Th with an NTM n sequence. Results: In to for NTM. Amo Conclusion: always under c

Research paper thumbnail of Cough in adolescent with cystic fibrosis, from nightmare to COVID-19 stigma: A qualitative thematic analysis

Journal of Pediatric Nursing, 2022

Purpose Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common... more Purpose Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common symptom. This study explored the experiences of adolescents with CF in Iran during the COVID-19 pandemic in relation to their cough. Design and methods In this qualitative study, we conducted 32 semi-structured interviews with 21 adolescents with CF. We analyzed the data thematically. Results We identified three main themes among adolescents with CF in relation to coughing: 1. Cough is a permanent companion; 2. Coughing raises fear of double stigma; 3. Patients' individualized coping strategies to deal with coughing. Participants complained that cough interrupted daily tasks and sleep, drew unwanted attention in public places, and elicited questions about whether they were COVID-19 patients or substance users—both highly stigmatized identities. Conclusion Although coughing is a protective mechanism for CF patients, frequent coughing often causes major challenges, particularly during the COVID pandemic, when people were acutely sensitive and aware about coughing. During the COVID-19 pandemic, in addition to taking care of themselves and managing the disease, CF patients therefore had to also overcome issues related to social stigma and isolation. Practice implications Healthcare workers play an important role in increasing public awareness about CF and its symptoms, including cough. During the pandemic, healthcare workers can help reduce the stigma of coughing through public education. Healthcare workers can actively communicate with patients to identify severe and ineffective cases of cough due to exacerbation of the disease and refer them to a specialist.

Research paper thumbnail of The correlation between 6-min walk test and respiratory parameters in children with cystic fibrosis

Irish Journal of Medical Science (1971 -), 2021

Background The 6-min walk test (6MWT) is a sub-maximal exercise test and has been widely used for... more Background The 6-min walk test (6MWT) is a sub-maximal exercise test and has been widely used for evaluating of exercise capacity of patients with cystic fibrosis (CF) in recent years. Few studies have examined the relationship between 6MWT and parameters used to assess the severity of the disease in children with CF. In this study, we have examined this relationship to find out if 6MWT can be a marker of the severity of cystic fibrosis. Methods A cross-sectional study was done to analyze the correlations among spirometry parameters, body mass index (BMI), chest tomography (CT), and 6MWT. CF patients, aged 7–14 years, were involved. Results Seventy-six patients, 32F/44M, mean age 10.49 ± 3.18 years, were studied. The mean distance in 6MWT was 447 ± 84.1. The following correlations versus distance were found: FEV1 ( r = 0.255, p = 0.026), FVC( r = 0.285, p = 0.013), FEF25–75% ( r = 0.546, p < 0.001), BMI ( r = 0.163, p = 0.160), and CT ( r = 0.075, p = 0.520).The following correlations versus O 2 saturation (SpO 2 ) decline were found: FEV1 ( r = −0.393, p < 0.001), FVC ( r = −0.431, p < 0.001), FEF25–75% ( r = −0.296, p = 0.010), BMI ( r = 0.042, p = 0.721), and CT ( r = −0.196, p = 0.090). There was a significant correlation between 6MWT (distance and SpO 2 decline) and pulmonary function test. There was no significant correlation between BMI, chest CT, and 6MWT. Conclusions 6MWT can be applied beside spirometry and chest CT for CF patients follow up.

Research paper thumbnail of Farber disease: report of three cases with joint involvement mimicking juvenile idiopathic arthritis

Journal of Musculoskeletal & Neuronal Interactions, 2019

Farber disease is a rare recessive autosomal disorder presented with three main features of joint... more Farber disease is a rare recessive autosomal disorder presented with three main features of joint involvement, subcutaneous nodules and hoarseness. Hereby we describe three new cases of Farber disease. All three cases were first misdiagnosed as juvenile idiopathic arthritis (JIA) due to the presentation of joint swelling. Addition of hoarseness and subcutaneous nodules to the initial joint swelling questioned the diagnosis of JIA and further evaluations led to the diagnosis of Farber disease. The first case was a 4-year old girl in whom a novel genetic mutation in ASAH1 gene was found. The second patient was a 4-year old girl presented with joint swelling at 7 month of age. The third patient was a 9-month boy complicated with severe respiratory distress. All patients were treated with symptomatic and supportive care. Two cases died due to respiratory failure and infection, but one patient follow up for 2 years after diagnosis. Farber disease should be considered as differential diag...

Research paper thumbnail of The Prevalence of Obesity, Technological Device Usage, Physical Activity and their Relationship with Spirometry Indicators among Children in Isfahan City, Iran

International Journal of Pediatrics, 2018

Background: The decrease in physical activities following increased usage of computer and digital... more Background: The decrease in physical activities following increased usage of computer and digital games has led to serious health consequences in children. This study investigates the prevalence of obesity, cellphone and computer usage and physical activity levels and their relationship with spirometry indicators in Iranian children. Materials and Methods: This is a cross-sectional study during 2013 to 2014 on high-school students in Isfahan, Iran. Sample size determined 1,690 students and sampling performed from 10 girls and 10 boy's high schools, based on multi-staged cluster randomized scheme. A research-made questionnaire was developed to complete by students interview and also contact with teachers and parents and measuring height and weight by researchers. If the asthma was probable (based symptoms and examined by physician) the spirometry was performed. Results: Overall 1,622 high-school students with the average age of 12.9 (+ 1.1) years were recruited. Prevalence of ob...

Research paper thumbnail of Genetic modification of cystic fibrosis with ΔF508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells

Iranian Journal of Basic Medical Sciences, 2021

Objective(s): Cystic fibrosis (CF) is an inherited autosomal recessive disease that is caused by ... more Objective(s): Cystic fibrosis (CF) is an inherited autosomal recessive disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The present study aimed to investigate the genetic modification of CF with ΔF508 mutation of the CFTR gene using CRISPR in peripheral blood mononuclear cells (PBMCs). Materials and Methods: Two single guide RNAs were designed to target sequences in the CFTR gene. The transfection efficiency of PBMC cells was examined through evaluation of green fluorescent protein (GFP) expression using fluorescent microscopy. Moreover, a sgRNA-Cas9 plasmid was tested to target the CFTR gene. The ΔF508 gene modification was evaluated and confirmed by PCR and Sanger sequencing methods. Results: Our results indicate the feasibility of site-specific gene targeting with the CRISPR/Cas9 system. 33% of the samples were corrected using CRISPR in mutant locus and confirmed by sequence blast at NCBI databases and primers outside the...

Research paper thumbnail of Infection Detection in Cystic Fibrosis Patients based on Tunable Q-factor Wavelet Transform of Respiratory Sound Signal and Ensemble Decision

Scientia Iranica, 2020

Most adult Cystic Fibrosis (CF) patients frequently suffer from Pseudomonas aeruginosa (PA) infec... more Most adult Cystic Fibrosis (CF) patients frequently suffer from Pseudomonas aeruginosa (PA) infection, which is strongly associated with inflammation, lung destruction, and increased mortality. Diagnosis of PA infection in the primary stage is essential to initiate the treatment and reduce the risk of chronic infection. Sputum culture is the gold standard for infection detection, but it is time-consuming. The objective of this study was to suggest and examine a method to decide about PA infection status in CF patients based only on their respiratory sound. Respiratory sounds were recorded from 36 CF patients. Some features which were generated from Tunable Qfactor wavelet transform(TQWT) components, were investigated. The features were fed into Support Vector Machine and also Ensemble classifier. The proposed method achieved an accuracy of 90.3% in identifying PA infection in CF patients. Furthermore, the probability of categorizing respiratory sounds as PA CF decreased significantly after the treatment of PA infection(P-value<0.003). Moreover, the method had a satisfactory performance in the presence of noises and artifacts. The developed method represents a novel approach to the diagnosis of PA infection in CF patients based only on respiratory sound signals, which is a necessary and innovative approach for early diagnosis of PA infection.

Research paper thumbnail of Nebulized Azithromycin Versus Oral Azithromycin as Anti-Inflammatory Therapy in Children with Cystic Fibrosis: A Prospective Randomized Open-Label Trial

Iranian Journal of Pediatrics, 2019

Background: Oral azithromycin is recommended as anti-inflammatory therapy for patients with cysti... more Background: Oral azithromycin is recommended as anti-inflammatory therapy for patients with cystic fibrosis (CF) 6 years of age and older to improve lung function and reduce exacerbations. Objectives: We evaluated the short-term efficacy and safety of nebulized azithromycin as anti-inflammatory therapy in children with CF chronically infected with Pseudomonas aeruginosa. Methods: In a randomized prospective study, children aged 8-18 years with clinically stable CF and FEV1 25%-75% predicted with no recent use of oral, intravenous or inhaled antipseudomonal antibiotics and azithromycin were randomized to receive either nebulized azithromycin (70 mg daily) or oral azithromycin 3 times per week for 28 days. Primary endpoint was changes in pulmonary function (FEV1). Secondary outcomes included changes in Pseudomonas aeruginosa (PA) colonization characteristics (count, phenotype), quality of life and weight. Results: A total of 280 patients were screened, of whom 60 were found eligible. Forty five patients (25 in the nebulized group) and 20 in the oral group) completed the study. After 28 days of treatment, nebulized azithromycin was significantly associated with improvements in FEV1 % predicted, quality of life measure and weight. Also PA count was significantly decreased. Importantly, improvements of FEV1 % predicted, quality of life, weight and the decline in PA count were statistically significantly greater with nebulized azithromycin compared with oral azithromycin. No significant difference was observed in PA phenotype in either treatment arms. Conclusions: Nebulized azithromycin is associated with a significant improvement in lung function, PA count, quality of life and weight in clinically stable CF children with chronic PA infection. The improvements observed were greater compared with oral azithromycin. Nebulized azithromycin could be suggested as a new therapeutic strategy for this life-limiting disease. Further clinical trials with novel nebulizer formulation of azithromycin and large number of participants are needed to further assess the efficacy, safety and sustained effect of this new therapeutic approach in children with CF.

Research paper thumbnail of Indications and Complications of Flexible Fiberoptic Bronchoscopy in Children: A 5-Year Experience at a Tertiary Care Hospital in Iran

Iranian Journal of Pediatrics, 2020

Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therap... more Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therapeutic modality in the evaluation of respiratory disorders in pediatric population. Objectives: The study aims to highlight common indications and risk of complications associated with bronchoscopy in our population. Methods: This retrospective evaluation was performed in all patients that underwent flexible bronchoscopy at Children's Medical Center (affiliated to Tehran University of Medical Sciences) between April 2011 and September 2016. Results: Of 800 bronchoscopies, 574 (71.7%) were performed for diagnostic and 226 (28.2%) for therapeutic purposes. Major indications included radiographic abnormalities (30%), a foreign body or suspected foreign body (28.5% of all FFBs) and stridor or wheezing (25% of all FBB). The incidence of major complications associated with FFBs was 0.87%. The most frequent complication was pneumothorax, followed by lung hemorrhage, and respiratory failure. Conclusions: Our findings support early intervention and utilization of bronchoscopy in the pediatric population with variable respiratory complaints.

Research paper thumbnail of Evaluation of adherence to medication treatment in pediatric patients with cystic fibrosis; a cross-sectional study

Cystic fibrosis, 2019

Background: Cystic fibrosis is a complex, multi-system impairment with poly pharmacy which affect... more Background: Cystic fibrosis is a complex, multi-system impairment with poly pharmacy which affects both children and their parents. Adherence to treatment is essential to optimize CF disease management. Aims and Objectives: The aim of the present study was to evaluate the medication adherence and indeed identify the factors affecting adherence in this population. Methods: An observational prospective study was conducted to recruited CF outpatient at the CF clinic of Children’s Medical Center in Iran. Children with CF and their parents were consecutively recruited. Baseline data, medical history, clinical outcome data, were collected via interview and review of medical notes. The Morisky Medication Adherence scale (MMAS) was used to measure adherence. Results: Three hundred patients with CF (age 1-25 years, mean 7.6 years, 58.7% males) and parent pairs were recruited. The mean score of MMAS in pulmonary, antibiotics, gastrointestinal and supplement medications was 5.1, 4.6, 4.1 and 3.3 respectively. According to the categorization of Morisky Scale, 58% of patients who received inhaler medications and 65% of patients who received antibiotics had a low adherence (lower than 6 scores). High adherence was associated with child age, parental higher educational degree and their beliefs, duration of illness and frequency of healthcare visit ((P=0.001). Conclusion: The results indicated that the non-adherence was very common in patients with CF. Our results suggest the need for educational interventions and planned patient monitoring program in patients with CF to optimize management and health outcomes.

Research paper thumbnail of Segment by segment lavage as an excellent alternative treatment in pulmonary alveolar proteinosis in a 3 years old boy

Research paper thumbnail of Is FEV1 associated with quality of life in children with cystic fibrosis? An original article

Immunopathologia Persa, 2019

Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life ... more Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis. Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44) were boys. The mean t...

Research paper thumbnail of First Cystic Fibrosis Patient Registry Annual Data Report-Cystic Fibrosis Foundation of Iran

ACTA MEDICA IRANICA, 2019

Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortali... more Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aimed to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced expiratory volume in one second (FEV1) was measured, and the comparative demographic, clinical, and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and severe (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%). Regarding the complex nature of CF and the necessity of constant monitoring of patients during the lifespan , the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients' data can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population.

Research paper thumbnail of Efficacy and safety of oral sildenafil in cystic fibrosis children with mild to moderate lung disease

Pediatric Pulmonology, 2019

Background: Airway inflammation due to chronic infection is the leading cause of respiratory fail... more Background: Airway inflammation due to chronic infection is the leading cause of respiratory failure and death in most of patients with cystic fibrosis (CF). There is some evidence about anti-inflammatory activity of phosphodiesterase inhibitors in adult patients with CF. This study was designed to evaluate the efficacy, safety, and tolerability of sildenafil (a phosphodiesterase inhibitor drug) in children with CF. Method: This uncontrolled before-after study was conducted on 20 children with CF (mean age 14 ± 2.8 years, 50% male) with mild to moderate lung disease who were referred to CF clinic of Imam Hossein hospital in Isfahan, Iran. The patients received oral sildenafil (1 mg/kg p.o tid for 3 months). Changes in spirometric values, maximal exercise capacity, and patient-reported health by using the cystic fibrosis questionnaire-revised (CFQ-R) were evaluated before and after treatment.

Research paper thumbnail of A new model-based framework for lung tissue segmentation in three-dimensional thoracic CT images

Signal, Image and Video Processing, 2017

A new framework for model-based lung tissue segmentation in three-dimensional thoracic CT images ... more A new framework for model-based lung tissue segmentation in three-dimensional thoracic CT images is proposed. In the first stage, a parametric model for lung segmenting surface is created using shape representation based on level sets method. This model is constituted by the sum of a mean distance function and a number of weighted eigenshapes. Consequently, unlike the other model-based segmentation methods, there is no need to specify any marker point in this model. In the second stage, the segmenting surface is varied so as to be matched with the binarized input image. For this purpose, a region-based energy function is minimized with respect to the parameters including the weights of eigenshapes and coefficients of a three-dimensional similarity transform.

Research paper thumbnail of LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells

Acta medica Iranica, 2016

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described... more LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to dat...

Research paper thumbnail of Primary ciliary dyskinesia in six patients with bronchiectasis

Pneumonologia i alergologia polska, 2016

Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. ... more Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and histor...